Conversely, NKCC1 expression in the striatum of several HD mouse models (R6/2 mice, N171-82Q and Hdh CAG knock-in mice) is abnormally elevated at the symptomatic stage (Y-T Hsu, Y-G Chang, Y-C Li, K-Y Wang, H-M Chen, D-J Lee, C-H Tsai, C-C Lien, Y Chern 2018, personal communication). Here, SLC12A2 is linked to Huntington disease.